Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The association of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) is thought to be rare, and its optimal treatment is unknown. Of 130 consecutive patients with a diagnosis of PBC, we identified 12 cases (9.2%) of overlap syndrome (10 females, 2 males; median age, 50 years) strictly defined by the presence of at least two of the three recognized biochemical, serological, and histological criteria of each disease. One patient had initially pure PBC and developed AIH characterized by a flare of alanine transaminase (ALT) (1,330 IU/L; N < 35), elevated immunoglobulin G (IgG) (42 g/L; N < 14.0), and presence of anti-smooth muscle antibodies (ASMA) after 20 months of ursodeoxycholic acid (UDCA) therapy. A complete clinical and biochemical remission was achieved under combination of corticosteroids and UDCA. Eleven patients had features of both diseases at presentation: high serum levels of alkaline phosphatase (AP) (median: 280 IU/L; N < 100), ALT (140 IU/L), and IgG (30.8 g/L), presence of mitochondrial antibodies (n = 9) or ASMA (n = 9), florid bile duct lesions (n = 8), and moderate or severe periportal or periseptal lymphocytic piecemeal necrosis (n = 11). UDCA (13-15 mg/kg/d) given alone in 5 patients induced a significant decrease in biochemical cholestasis but not in ALT levels, and liver fibrosis progressed in 3 patients. Corticosteroids given alone in 6 patients induced a significant decrease in ALT, IgG, and AP levels, but none had a biochemical normalization. The patients with persistently abnormal liver tests under either UDCA or corticosteroids received both UDCA and corticosteroids. A further marked biochemical improvement was observed, and all patients became asymptomatic. We conclude that, in patients with PBC: 1) overlap syndrome with AIH is not rare; 2) flares of AIH may occur either spontaneously or under UDCA; and 3) combination of UDCA and corticosteroids is required in most patients to obtain a complete biochemical response. Overlap syndrome may represent an important and unrecognized cause of resistance to UDCA in patients with PBC.
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PMID:Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. 969 90

A 54-year-old woman was admitted to our hospital because of acute liver injury. Since she had a history of having used a diet product, drug-induced liver injury (DILI) was initially considered. However, the patient was subsequently diagnosed as suffering from primary biliary cirrhosis (PBC) based on the findings of liver histology and serum anti-mitochondrial antibody positivity. Overlap syndrome between PBC and autoimmune hepatitis (AIH) was also suspected, however, serum levels of aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase became normal three months later, after treatment with combination therapy comprising ursodeoxycholic acid plus bezafibrate. We therefore concluded that the liver disease in this patient was actually PBC, but that it resembled overlap syndrome or DILI. In cases of PBC, a rapid onset, as frequently seen in the case of DILI, viral hepatitis or AIH, is not common. We herein report a rare case of PBC which resembled DILI.
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PMID:Rapid-onset primary biliary cirrhosis resembling drug-induced liver injury. 1629 9

Overlap syndrome in Autoimmune liver disease is not unusual but the switch over from one type of autoimmune liver disease to another is not well recognized. We report 2 cases of primary biliary cirrhosis (PBC) who with time, crossed over to autoimmune hepatitis (AIH). Recognition of such switch over from PBC to AIH is important for appropriate change in management of the patients.
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PMID:Primary biliary cirrhosis and autoimmune hepatitis switch over: report of 2 cases. 1651 63

In this article, the authors use the term "overlaps" to refer to the coexistence of primary biliary cirrhosis (PBC) with another autoimmune condition that involves the liver or extrahepatic organs. Diagnosing PBC-autoimmune hepatitis (PBC-AIH) overlap syndrome remains a challenge, especially because there is still no consensus on the most appropriate diagnostic criteria. The prevalence of this condition varies considerably among series of PBC patients, and its treatment demands a combination of ursodeoxycholic acid and immunosuppressive drugs. Overlap syndrome between PBC and primary sclerosing cholangitis is described in exceptional cases. About one in three PBC patients have a concomitant extrahepatic autoimmune disease, which may include rheumatological, endocrinological, gastrointestinal, pulmonary, or dermatological conditions. Overlaps raise several questions, about whether they share much the same genetic susceptibility, as is generally assumed. The pathogenesis of these conditions includes the production of both innate and adaptive immune responses targeting cholangiocytes as well as different extrahepatic tissues. In this sense, overlaps in PBC represent a continuous spectrum of autoimmunity involving liver and extrahepatic tissues.
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PMID:Primary biliary cirrhosis: overlaps with other autoimmune disorders. 2505 58

The article contains the description of a clinical case of the patient P. of 59 years with autoimmune pancreatitis (AIP) associated with a sclerousis cholangitis (SC), and the analysis of literature on this problem. The patient was operated (cholecystenterostomy, enteroenterostomy) for pancreatic cancer. The pancreatic cancer was excluded. Overlap syndrome (primary biliary cirrhosis/autoimmune hepatitis), pseudotumorosis pancreatitis were excluded during the period of monitoring in hospitals of Moscow. In our clinic the diagnosis AIP is established on the basis of Mayo's criterion (HISORt: Histology, Imaging, Serology, Other organ involvement, Response to corticosteroid therapy). The increased level of immunoglobulin G (IgG) by 3 times (46,2 g/l, norm--6.58-18.37 g/l), IgG4 by 25 times (49.5 g/I, norm--0.33-2.01 g/l) was revealed. MRI of abdominal organs showed diffusion increase pancreas with a peripheral hypointensive rim, local stenosis of the Major pancreatic duct in the head pancreas. Immune histochemical study surgical biopsy of pancreas showed large number of IgG-positive plasma cells. Glucocorticosteroids (GCS, prednisolone 40 mg/d), ursodeoxycholic acid 1000 mg/d, creon 120000 ed/d promoted improvement of clinical picture AIP and decrease IgG4 (to 6.4 g/I). After 12 months IgG4 wasn't defined. Control MRI revealed dilatation small biliary ducts in 8, 5, 4 liver segments and thickening wall common bile duct (to 2 mm) at large distance. The peculiarity of the case--the 15-year follow-up of patients with AlP who had inappropriate surgery. Late diagnosis and delayed treatment GCS adversely affected the course of the disease, which led to the development of secondary biliary cirrhosis.
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PMID:[SCLEROUSIS CHOLANGITIS AT THE AUTOIMMUNE PANCREATITIS PATIENT]. 2681 26

Overlap syndrome between primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) is extremely rare in Japan. We herein report two adult patients with PSC-AIH overlap syndrome. They were diagnosed with PSC-AIH overlap syndrome based on the findings of endoscopic retrograde cholangiography and liver biopsy, and using the International Autoimmune Hepatitis Group scoring system. In both cases, PSC preceded AIH, and combination therapy with steroid and ursodeoxycholic acid was effective. Because there are few reported cases in Japan, it is important to study more cases to shed light on the clinical and pathological features of PSC-AIH overlap syndrome.
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PMID:Two Cases of Primary Sclerosing Cholangitis Overlapping with Autoimmune Hepatitis in Adults. 2825 Feb 96

Overlap syndrome indicates the coexistence of 2 or more autoimmune liver diseases in the same individual, occurring simultaneously or sequentially. Cases of overlap of autoimmune hepatitis (AIH) with primary biliary cholangitis (PBC) and of AIH with primary sclerosing cholangitis (PSC) are known and have defined criteria for diagnosis. Overlap between PBC and PSC has been reported in only a few case reports. The cause for the rarity of this entity compared to other overlap syndromes is unclear. We present a case of an overlap syndrome of PBC with PSC in a 35-year-old woman.
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PMID:Overlap Syndrome between Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. 3003 26

Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) is typically defined as concomitant or serial presentation with clinical features of both of these 2 distinct diseases. The Paris criteria and variations of the International Autoimmune Hepatitis group scoring systems for the diagnosis of AIH have been used to diagnose overlap syndrome. If left untreated, patients with overlap syndrome will have higher rates of portal hypertension, gastrointestinal bleeding, ascites, death, and need for liver transplant. Therefore, early identification is essential in providing appropriate therapy to potentially prevent long-term adverse outcomes in patients with overlap syndrome.
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PMID:Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis. 3025 56